Splenic marginal zone lymphoma (SMZL) is a rare lymphoproliferative disease involving B-cells and affecting elderly patients. SMZL plague peripheral blood and bone marrow, spleen. Lymph nodes are generally spared. SMZL is due to a protracted antigen stimulation of B lymphocytes and of microenvironment leading B-cell to polyclonal and then oligoclonal/monoclonal growth, promoting lymphoproliferation. Integration of the NOTCH2 and NFk-B signaling has been recently identified as the primary mechanism of neoplastic proliferation in SMZL. In total 20% of cases carry mutations in NOTCH2. Although SMZL has an indolent course, progression to diffuse large B-cell lymphoma occurs in about 10-15% of patients. Establishing the prognosis is a key step in disease management, depending on both individual risk and patients' health status. This review discusses tailored treatment of SMZL patients. Progression risk factors include nodal and extra-nodal involvement, peripheral lymphocytosis, anemia and thrombocytopenia. Patients with two or more score points have a median survival of <5 years. Watch and wait strategy is appropriate in low-risk and asymptomatic patients, whereas treatment of symptomatic patients ranges from splenectomy to rituximab monotherapy or associated with chemotherapy.

中文翻译：

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脾边缘区淋巴瘤的风险定制治疗。

脾边缘区淋巴瘤 (SMZL) 是一种罕见的涉及 B 细胞的淋巴细胞增殖性疾病，影响老年患者。SMZL 治鼠疫末梢血及骨髓、脾脏。淋巴结一般不会受到影响。SMZL 是由于 B 淋巴细胞和微环境的长期抗原刺激导致 B 细胞多克隆，然后寡克隆/单克隆生长，从而促进淋巴细胞增殖。NOTCH2 和 NFk-B 信号传导的整合最近被确定为 SMZL 肿瘤增殖的主要机制。总共 20% 的病例携带 NOTCH2 突变。尽管 SMZL 病程呈惰性，但约 10-15% 的患者会进展为弥漫性大 B 细胞淋巴瘤。确定预后是疾病管理的关键步骤，具体取决于个体风险和患者的健康状况。这篇综述讨论了 SMZL 患者的定制治疗。进展危险因素包括淋巴结和结外受累、外周淋巴细胞增多、贫血和血小板减少。具有两个或多个评分点的患者的中位生存期<5年。观察和等待策略适用于低风险和无症状患者，而有症状患者的治疗范围从脾切除术到利妥昔单抗单药治疗或联合化疗。